Coalition for Global Hearing Health Hearing Care Pathways Working Group: Guidelines for Clinical Guidance for Readiness and Development of Evidence-Based Early Hearing Detection and Intervention Programs.

Editor's Note: The following article discusses the timely topic Clinical Guidance in the areas of Evidence-Based Early Hearing Detection and Intervention Programs. This article aims to discuss areas of services needed, guidance to countries/organizations attempting to initiate early hearing detection and intervention systems. Expert consensus and systematic/scoping reviews were combined to produce recommendations for evidence-based clinical practice. In Ear and Hearing, our long-term goal for the Point of View article is to stimulate the field's interest in and to enhance the appreciation of the author's area of expertise. Hearing is an important sense for children to develop cognitive, speech, language, and psychosocial skills. The goal of universal newborn hearing screening is to enable the detection of hearing loss in infants so that timely health and educational/therapeutic intervention can be provided as early as possible to improve outcomes. While many countries have implemented universal newborn hearing screening programs, many others are yet to start. As hearing screening is only the first step to identify children with hearing loss, many follow-up services are needed to help them thrive. However, not all of these services are universally available, even in high-income countries. The purposes of this article are (1) to discuss the areas of services needed in an integrated care system to support children with hearing loss and their families; (2) to provide guidance to countries/organizations attempting to initiate early hearing detection and intervention systems with the goal of meeting measurable benchmarks to assure quality; and (3) to help established programs expand and improve their services to support children with hearing loss to develop their full potential. Multiple databases were interrogated including PubMed, Medline (OVIDSP), Cochrane library, Google Scholar, Web of Science and One Search, ERIC, PsychInfo. Expert consensus and systematic/scoping reviews were combined to produce recommendations for evidence-based clinical practice. Eight essential areas were identified to be central to the integrated care: (1) hearing screening, (2) audiologic diagnosis and management, (3) amplification, (4) medical evaluation and management, (5) early intervention services, (6) family-to-family support, (7) D/deaf/hard of hearing leadership, and (8) data management. Checklists are provided to support the assessment of a country/organization's readiness and development in each area as well as to suggest alternative strategies for situations with limited resources. A three-tiered system (i.e., Basic, Intermediate, and Advanced) is proposed to help countries/organizations at all resource levels assess their readiness to provide the needed services and to improve their integrated care system. Future directions and policy implications are also discussed.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Support Principles.

This article is the sixth in a series of eight articles that comprise a special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. The Support Principles article is the second of three articles that describe the 10 Principles of FCEI-DHH, preceded by the Foundation Principles, and followed by the Structure Principles, all in this special issue. The Support Principles are composed of four Principles (Principles 3, 4, 5, and 6) that highlight (a) the importance of a variety of supports for families raising children who are DHH; (b) the need to attend to and ensure the well-being of all children who are DHH; (c) the necessity of building the language and communication abilities of children who are DHH and their family members; and (d) the importance of considering the family's strengths, needs, and values in decision-making.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Cultural & Global Implications.

This article is the third in a series of eight articles that comprise this special issue on family-centered early intervention for children who are deaf or hard of hearing and their families (FCEI-DHH). It highlights the origins of FCEI-DHH in Western contexts and well-resourced locations and emphasizes the role of culture(s) in shaping FCEI-DHH. This article also cautions against the direct application of the 10 FCEI-DHH Principles presented in this issue across the globe without consideration of cultural implications. Cultural perceptions of decision-making processes and persons who can be decision-makers in FCEI-DHH are explored. Deaf culture(s) and the benefits of exposure to DHH adults with diverse backgrounds are introduced. Structural inequities that impact families' access to FCEI-DHH programs/services and systems, within and among nations and regions, are noted. The need to consider the cultural influences on families is emphasized; this applies to all levels of FCEI, including the development of systems through implementation of supports.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Foundation Principles.

This article is the fifth in a series of eight articles that comprise a special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. The 10 FCEI-DHH Principles are organized conceptually into three sections (a) Foundation Principles, (b) Support Principles, and (c) Structure Principles. Collectively, they describe the essential Principles that guide FCEI for children who are DHH and their families. This article describes the Foundation Principles (Principles 1 and Principle 2). The Foundation Principles emphasize the essential elements of ensuring that families with children who are DHH can access early intervention (EI) and other appropriate supports, as well as highlight the need for provision of EI that is family-centered. Implementation of these FCEI-DHH Principles is intended to improve the lives and the outcomes of children who are DHH and their families around the globe.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Structure Principles.

This article is the seventh in a series of eight articles that comprise a special issue on family-centered early intervention for children who are deaf or hard of hearing and their families, or FCEI-DHH. This article, Structure Principles, is the third of three articles (preceded by Foundation Principles and Support Principles) that describe the 10 FCEI-DHH Principles. The Structure Principles include 4 Principles (Principle 7, Principle 8, Principle 9, and Principle 10) that highlight (a) the importance of trained and effective Early Intervention (EI) Providers, (b) the need for FCEI-DHH teams to work collaboratively to support families, (c) the considerations for tracking children's progress through developmental assessment, and (d) the essential role of progress monitoring to continuously improve systems.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Guiding Values.

This article is the second of eight articles in this special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. Five foundational values that guide FCEI-DHH are described, providing an evidence-informed, conceptual context for the 10 FCEI-DHH Principles and other articles presented in this issue. These values are applicable for Early Intervention (EI) Providers and other professionals on FCEI teams, as well as for FCEI-DHH programs/services and systems. The five key values include (1) being family-centered, (2) responding to diversity, (3) involving invested parties, especially families and individuals who are DHH, (4) supporting holistic child development, and (5) ensuring fundamental human rights. These evidence-informed values are considered essential to the effective provision of FCEI-DHH supports.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Introduction.

This article is the first of eight articles in this special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH), or FCEI-DHH. In 2013, a diverse panel of experts published an international consensus statement on evidence-based Principles guiding FCEI-DHH. Those original Principles have been revised through a coproduction process involving multidisciplinary collaborators and an international consensus panel, utilizing the best available evidence and current understanding of how to optimally support children who are DHH and their families. This revision (referred to as expanded Principles) was motivated by the need to incorporate (a) input from family leaders and DHH leaders, (b) broader international and cultural perspectives, (c) new empirical evidence, and (d) research in human development. This Introduction provides an overview of the rationale, purposes, and main content areas to be addressed throughout the special issue.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Call to Action.

This Call to Action is the eighth and final article in this special issue on Family-Centered Early Intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, or FCEI-DHH. Collectively, these articles highlight evidence-informed actions to enhance family well-being and to optimize developmental outcomes among children who are DHH. This Call to Action outlines actionable steps to advance FCEI-DHH supports provided to children who are DHH and their families. It also urges specific actions to strengthen FCEI-DHH programs/services and systems across the globe, whether newly emerging or long-established. Internationally, supports for children who are DHH are often siloed, provided within various independent sectors such as health/medicine, education, early childhood, and social and disability services. With this Call to Action, we urge invested parties from across relevant sectors to join together to implement and improve FCEI-DHH programs/services and systems, build the capacity of early intervention (EI) Providers and other professionals, extend research regarding FCEI-DHH, and fund EI supports, systems, and research, all with the aim of advancing outcomes for families and their children who are DHH.

Family-Centered Early Intervention Deaf/Hard of Hearing (FCEI-DHH): Methods.

This is the fourth article in a series of eight that comprise a special issue on family-centered early intervention (FCEI) for children who are deaf or hard of hearing (DHH) and their families, FCEI-DHH. This article describes the co-production team and the consensus review method used to direct the creation of the 10 Principles described in this special issue. Co-production is increasingly being used to produce evidence that is useful, usable, and used. A draft set of 10 Principles for FCEI-DHH and associated Tables of recommended behaviors were developed using the knowledge creation process. Principles were refined through two rounds of eDelphi review. Results for each round were analyzed using measures of overall group agreement and measures that indicated the extent to which the group members agreed with each other. After Round 2, with strong agreement and low to moderate variation in extent of agreement, consensus was obtained for the 10 Principles for FCEI-DHH presented in this special issue. This work can be used to enhance evolution of FCEI-DHH program/services and systems world-wide and adds to knowledge in improvement science.

Speech Discrimination in Infancy Predicts Language Outcomes at 30 Months for Both Children with Normal Hearing and Those with Hearing Differences.

BACKGROUND: Speech discrimination assessments are used to validate amplification fittings of older children who are hard of hearing (CHH). Unfortunately, speech discrimination is not assessed clinically ≤24 months and in turn no studies have investigated the relationship between speech discrimination during infancy and later language development among CHH. OBJECTIVE: To examine the relationship between an individual infant's speech discrimination measured at 9 months and their expressive/receptive spoken language at 30 months for children with normal hearing (CNH) and CHH. METHODS: Behavioral speech discrimination was assessed at 9 months and language assessments were conducted at 16, 24, and 30 months using a parent questionnaire, and at 30 months using the Mullen Scales of Early Learning among 90 infants (49 CNH; 41 CHH). RESULTS: Conditioned Head Turn (CHT) performance for /a-i/ significantly predicted expressive and receptive language at 30 months across both groups. Parental questionnaires were also predictive of later language ability. No significant differences in speech discrimination or language outcomes between CNH and CHH were found. CONCLUSIONS: This is the first study to document a positive relationship between infant speech discrimination and later language abilities in both early-identified CHH and CNH.

Clinical evaluation and etiologic diagnosis of hearing loss: A clinical practice resource of the American College of Medical Genetics and Genomics (ACMG).

Hearing loss is a common and complex condition that can occur at any age, can be inherited or acquired, and is associated with a remarkably wide array of etiologies. The diverse causes of hearing loss, combined with the highly variable and often overlapping presentations of different forms of hearing loss, challenge the ability of traditional clinical evaluations to arrive at an etiologic diagnosis for many deaf and hard-of-hearing individuals. However, identifying the etiology of hearing loss may affect clinical management, improve prognostic accuracy, and refine genetic counseling and assessment of the likelihood of recurrence for relatives of deaf and hard-of-hearing individuals. Linguistic and cultural identities associated with being deaf or hard-of-hearing can complicate access to and the effectiveness of clinical care. These concerns can be minimized when genetic and other health care services are provided in a linguistically and culturally sensitive manner. This clinical practice resource offers information about the frequency, causes, and presentations of hearing loss and suggests approaches to the clinical and genetic evaluation of deaf and hard-of-hearing individuals aimed at identifying an etiologic diagnosis and providing informative and effective patient education and genetic counseling.

Frequency of Early Intervention Sessions and Vocabulary Skills in Children with Hearing Loss.

BACKGROUND: A primary goal of early intervention is to assist children in achieving age-appropriate language skills. The amount of intervention a child receives is ideally based on his or her individual needs, yet it is unclear if language ability impacts amount of intervention and/or if an increased frequency of intervention sessions results in better outcomes. The purpose of this study was to determine the relationship between the frequency of early intervention sessions and vocabulary outcomes in young children with hearing loss. METHODS: This was a longitudinal study of 210 children 9 to 36 months of age with bilateral hearing loss living in 12 different states. Expressive vocabulary skills were evaluated using the MacArthur-Bates Communicative Development Inventories. RESULTS: A higher number of intervention sessions reported at the first assessment predicted better vocabulary scores at the second assessment, and more sessions reported at the second assessment predicted better scores at the third assessment. For each increase in the number of sessions reported, there was a corresponding, positive increase in vocabulary quotient. In contrast, children's vocabulary ability at an earlier time point did not predict intervention session frequency at a later point in time. CONCLUSIONS: A significant prospective effect was apparent with more therapy sessions resulting in improved vocabulary scores 9 months later. These findings underscore the importance of early intervention. Pediatricians and other health care professionals can help apply these findings by counseling parents regarding the value of frequent and consistent participation in early intervention.

Reading Proficiency Trends Following Newborn Hearing Screening Implementation.

OBJECTIVES: To investigate trends in population-level school-aged reading scores among students with hearing loss in an urban Colorado school district after implementation of universal newborn hearing screening (UNHS) and Early Hearing Detection and Intervention. METHODS: The final sample included 1422 assessments conducted during the 2000-2001 through 2013-2014 school years for 321 children with hearing loss in grades 3 through 10. Longitudinal hierarchical linear modeling analyses were used to examine reading proficiency (controlling for birth year, grade in school, free and reduced lunch status, additional disability services, and English not spoken in the home). The Colorado Student Assessment Program was administered to students in third through 10th grades throughout the state. The test years chosen included children born before and after implementation of UNHS. RESULTS: After implementation of UNHS, significant longitudinal reading proficiency improvements were observed by birth year and grade overall and for all subgroups. However, gains in reading proficiency were substantially less for children eligible for free and reduced lunch and those with moderate-severe to profound hearing loss. With each succeeding birth cohort and grade, increased numbers of children participated in testing because of improved language skills, with higher proportions identified as proficient or advanced readers. CONCLUSIONS: Notable improvements in reading proficiency after Early Hearing Detection and Intervention implementation were demonstrated, as all groups of children with hearing loss became more likely to achieve proficient and advanced reading levels. On the other hand, some disparities increased, with greater improvements in reading proficiency for children in economically advantaged families.

Outcomes of Universal Newborn Screening Programs: Systematic Review.

BACKGROUND: This systematic review examined the outcomes (age of identification and intervention, developmental outcomes, cost-effectiveness, and adverse effects on parents) of universal newborn hearing screening (UNHS) for children with permanent congenital hearing loss (PCHL). MATERIALS AND METHODS: Multiple electronic databases were interrogated in March and April 2020 with further reports identified from article citations and unpublished literature. UNHS reports in English with comparisons of outcomes of infants who were not screened, and infants identified through other hearing screening programs. RESULTS: 30 eligible reports from 14 populations with 7,325,138 infants screened through UNHS from 1616 non-duplicate references were included. UNHS results in a lower age of identification, amplification, and the initiation of early intervention services and better language/literacy development. Better speech perception/production were shown in younger, but not in older, children with early identification after UNHS. No significant findings were found for behavior problems and quality of life. UNHS was found to be cost-effective in terms of savings to society. In addition, no significant parental harm was noted as a result of UNHS. CONCLUSIONS: In highly developed countries, significantly better outcomes were found for children identified early through UNHS programs. Early language development predicts later literacy and language development.

Do Acoustic Environment Characteristics Affect the Lexical Development of Children With Cochlear Implants? A Longitudinal Study Before and After Cochlear Implant Activation.

Purpose This study investigates the acoustic environment of children with cochlear implants (CIs) and the relationship between exposure to speech, in noise and in quiet, and the children's lexical production up to 1 year after CI activation, while controlling for the effect of early individual differences in receptive vocabulary growth. Method Eighteen children with CIs were observed at 3, 6, and 12 months after CI activation. Children's spontaneous word production during interaction with their mothers (types and tokens) and their expressive and receptive vocabulary size were considered. The characteristics of the acoustic environments in terms of acoustic scenes (speech in noise or in quiet, quiet, noise, music, and other) and of loudness ranges were assessed using data logging of the children's devices. Results Data analysis showed that both the number of tokens and the number of types produced 1 year after CI activation were affected by the children's exposure to speech in quiet with a loudness range between 40 and 69 dB. Expressive vocabulary size and types were affected by the receptive vocabulary knowledge that the children achieved over the first 3 months after CI activation. Conclusions Our data support the role of speech environment and individual differences in early comprehension on lexical production. The importance of exposure to speech with particular characteristics for the lexical development of children with CIs and the implications for clinical practice are discussed.

Early Intervention, Parent Talk, and Pragmatic Language in Children With Hearing Loss.

BACKGROUND AND OBJECTIVES: Pragmatic language skills form the foundation for conversational competence, whereas deficits in this area are associated with behavioral problems and low literacy skills. Children who are deaf or hard of hearing demonstrate significant delays in this critical area of language. Our purpose with this research was to identify variables associated with pragmatic language ability in children who are deaf or hard of hearing. METHODS: This was a longitudinal study of 124 children with bilateral hearing loss between 4 and 7 years of age living in Colorado. As part of a comprehensive speech and language assessment, pragmatic language skills were evaluated annually by using the Pragmatics Checklist. RESULTS: The children's pragmatic skills increased significantly with age. Higher levels of pragmatic language ability at 7 years of age were predicted by (1) meeting Early Hearing Detection and Intervention 1-3-6 guidelines (hearing screening by 1 month, identification of hearing loss by 3 months, and receiving intervention by 6 months of age), (2) greater quantity of parent talk, (3) higher nonverbal intelligence, (4) lesser degrees of hearing loss, and (5) higher maternal education. CONCLUSIONS: With the findings of this study, we underscore the importance of pediatricians and other health care professionals counseling parents about the value of adherence to the Early Hearing Detection and Intervention 1-3-6 guidelines with regard to intervention outcomes. The strong association between amount of child-directed parent talk in the first 4 years of life and pragmatic language outcomes at 7 years of age emphasizes the need for professionals to encourage parents to talk to their children as much as possible.

Use of the LENA Autism Screen with Children who are Deaf or Hard of Hearing.

Background and Objectives: This systematic review reports the evidence from the literature concerning the potential for using an automated vocal analysis, the Language ENvironment Analysis (LENA, LENA Research Foundation, Boulder, CO, USA) in the screening process for children at risk for autism spectrum disorder (ASD) and deaf or hard of hearing (D/HH). ASD and D/HH have increased comorbidity, but current behavioral diagnostic and screening tools have limitations. The LENA Language Autism Screen (LLAS) may offer an additional tool to disambiguate ASD from D/HH in young children. Materials and Methods: We examine empirical reports that use automatic vocal analysis methods to differentiate disordered from typically developing children. Results: Consensus across the sampled scientific literature shows support for use of automatic methods for screening and disambiguation of children with ASD and D/HH. There is some evidence of vocal differentiation between ASD, D/HH, and typically-developing children warranting use of the LLAS, but additional empirical evidence is needed to better understand the strengths and weaknesses of the tool. Conclusions: The findings reported here warrant further, more substantive, methodologically-sound research that is fully powered to show a reliable difference. Findings may be useful for both clinicians and researchers in better identification and understanding of communication disorders.

Predictors of Vocabulary Outcomes in Children Who Are Deaf or Hard of Hearing From Spanish-Speaking Families.

Purpose The goal of this study was to identify predictors of expressive vocabulary in young Spanish-speaking children who are deaf or hard of hearing living in the United States. Method This cross-sectional study considered 53 children with bilateral hearing loss between 8 and 34 months of age ( M = 24, SD = 6.9). Demographic variables, variables related to the hearing loss, and intervention variables were included in a hierarchical regression analysis to predict expressive vocabulary quotients from the MacArthur Inventario del Desarrollo de Habilidades Comunicativas (Communicative Development Inventories; Jackson-Maldonado et al., 2003 ). Results Chronological age, degree of hearing loss, functional hearing ability ratings, age of enrollment in early intervention, and the interaction between chronological age and age of intervention accounted for 61.5% of the vocabulary variance. Children who received intervention by 6 months of age achieved significantly higher vocabulary outcomes than children who started intervention later. Conclusion The children's mean vocabulary outcomes were below average when compared with hearing peers. This was especially true for older children, children with moderately-severe-to-profound hearing loss, and children who began intervention after 6 months of age. This delay in vocabulary outcomes has the potential to interfere with future reading and academic outcomes.

Language Outcomes Improved Through Early Hearing Detection and Earlier Cochlear Implantation.

HYPOTHESIS: Early identification and intervention, earlier cochlear implantation, and mother's level of education will directly and/or indirectly impact the language outcomes of children with cochlear implants (CIs). BACKGROUND: Identifying factors that contribute to the wide range of language outcomes in children who use CIs will assist healthcare and rehabilitation professionals in optimizing service delivery for this population. Universal newborn hearing screening provides an opportunity to examine the relationship between meeting the early hearing detection and intervention (EHDI) 1-3-6 guidelines and child language outcomes. These guidelines recommend screening by 1 month, confirmation of hearing loss by 3 months, and intervention by 6 months of age. METHODS: Participants were 125 children with CIs ranging from 13 to 39 months of age. Language ability was measured using the Child Development Inventory and MacArthur-Bates Communicative Development Inventories. RESULTS: Meeting EHDI 1-3-6, higher levels of maternal education and earlier cochlear implant activation had a direct, positive impact on language outcomes. Meeting the EHDI 1-3-6 guidelines also had an indirect positive effect on language outcomes via increasing the probability that the children's CIs would be activated earlier. Maternal education did not significantly predict age of cochlear implant activation nor whether a child met EHDI 1-3-6. CONCLUSION: Ensuring families meet the EHDI 1-3-6 guidelines is an early step that can lead to higher language outcomes and also earlier cochlear implantation.